SCLEROMYXEDEMA WITH SYSTEMIC INVOLVEMENT MIMICS RHEUMATIC DISEASES

Scleromyxedema is an infiltrative skin disease produced by hyaluronic acid deposition in the dermis. A benign monoclonal gammopathy is usually present. We report 2 patients with scleromyxedema and systemic illnesses. Both patients had muscle weakness, dysphagia, and weight loss in addition to the skin changes. One also had sclerodactyly, telangiectasias, and Raynaud’s phenomenon. Scleromyxedema with systemic involvement may mimic rheumatic diseases. Scleromyxedema, also known as papular mucinosis or lichen myxedematosus, is an unusual connective tissue disease which is characterized by infiltrative skin lesions and a monoclonal paraprotein. Patients with this disorder rarely have systemic manifestations (14). We report 2 cases of scleromyxedema with systemic features which mimicked rheumatic diseases. Case reports. Patient I. Patient 1, a 45-year-old white man, was in excellent health until 1976, when he developed dyspnea on exertion. Shortly thereafter, he noted the appearance of small white papules over his forearms and the extensor surfaces of his fingers, tightening of the skin around his hands and face, and Raynaud’s phenomenon which was precipitated by cold and involved triphasic color changes. The skin